Impaired LEF1 Activation Accelerates iPSC-Derived Keratinocytes Differentiation in Hutchinson-Gilford Progeria Syndrome.

Hutchinson-Gilford progeria syndrome (HGPS) is a detrimental premature aging disease caused by a point mutation in the human gene. This mutation results in the abnormal accumulation of a truncated pre-lamin A protein called progerin. Among the drastically accelerated signs of aging in HGPS patients, severe skin phenotypes such as alopecia and sclerotic skins always develop with the disease progression.

Mechanisms of angiogenic incompetence in Hutchinson-Gilford progeria via downregulation of endothelial NOS.

Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder with features of accelerated aging. The majority of HGPS cases are caused by a de novo point mutation in the LMNA gene (c.1824C>T; p.

Ultraviolet radiation protection potentials of Methylene Blue for human skin and coral reef health.

Methylene blue (MB) is a century-old medicine, a laboratory dye, and recently shown as a premier antioxidant that combats ROS-induced cellular aging in human skins. Given MB's molecular structure and light absorption properties, we hypothesize that MB has the potential to be considered as a sunscreen active for UV radiation protection. In this study, we tested the effects of MB on UVB ray-induced DNA double-strand breaks in primary human keratinocytes.

In vivo base editing rescues Hutchinson-Gilford progeria syndrome in mice.

Hutchinson-Gilford progeria syndrome (HGPS or progeria) is typically caused by a dominant-negative C•G-to-T•A mutation (c.1824 C>T; p.G608G) in LMNA, the gene that encodes nuclear lamin A.

Peroxisomal abnormalities and catalase deficiency in Hutchinson-Gilford Progeria Syndrome.

Peroxisomes are small, membrane-enclosed eukaryotic organelles that house various enzymes with metabolic functions. One important feature in both Hutchinson-Gilford Progeria Syndrome (HGPS) and normal aging is the elevated levels of Reactive Oxygen Species (ROS), which are generated from metabolic pathways with the capacity to cause oxidative damage to macromolecules within the cells. Although peroxisomal bioreactions can generate free radicals as their byproducts, many metabolic enzymes within the peroxisomes play critical roles as ROS scavengers, in particular, catalase.

Transient induction of telomerase expression mediates senescence and reduces tumorigenesis in primary fibroblasts.

Telomerase is an enzymatic ribonucleoprotein complex that acts as a reverse transcriptase in the elongation of telomeres. Telomerase activity is well documented in embryonic stem cells and the vast majority of tumor cells, but its role in somatic cells remains to be understood. Here, we report an unexpected function of telomerase during cellular senescence and tumorigenesis.

Hierarchically porous poly(ethylenimine) modified poly(styrene-co-divinylbenzene) microspheres for the adsorption of gold nanoparticles and simultaneously being transformed as the nanoparticles immobilized catalyst.

With the extensive applications of gold nanoparticles (AuNPs) and the confirmation of their toxicity on human health and environment, it was urgent to remove AuNPs from environment. The hierarchically porous poly(ethylenimine) modified poly(styrene-co-divinylbenzene) microsphere (PEI-PS-DVB) was prepared and characterized by scanning electron microscopy, X-ray diffraction, transform infrared spectrometry, energy dispersive X-ray spectrometry, elemental analysis, contact angle, zeta potential analysis, N adsorption-desorption and mercury intrusion porosimetry. PEI-PS-DVB possessed abundant flow-through pores (70-120 nm) and meso/micropores (<50 nm); the former pores enabled full availability of the adsorbent to relatively large adsorbate, i.

Flow-through silica: A potential matrix for fast chromatographic enantioseparation with high enantioselectivity.

The demand for fast chromatographic enantioseparation aroused the hot research in stationary phase matrix. In the present study, the flow-through silica, which is characterized by hierarchical pores of through pores in several hundred nanometer range and mesopores about 20nm, was attempted for fast enantioseparation. Thanks to the large surface area and full openness of the through pores, the flow-through silica had comparable cellulose derivative loading amount as the commercial wide-pore silica, which was impracticable for most of the core-shell particles and sub-2-μm fully porous silica.

Biotransformation of NSAIDs by pig liver microsomes in vitro: Kinetics, metabolites identification and toxicity prediction.

Non-steroidal anti-inflammatory drugs (NSAIDs) are one of the most frequently used pharmaceuticals in animals. In the current study, the biotransformation of five NSAIDs by pig liver microsomes (PLMs) was studied. The pseudo-first-order kinetics mode was obtained for the metabolization of the studied NSAIDs by PLMs in vitro.

FGFR2 mutations in bent bone dysplasia syndrome activate nucleolar stress and perturb cell fate determination.

Fibroblast Growth Factor (FGF) signaling promotes self-renewal in progenitor cells by encouraging proliferation and inhibiting cellular senescence. Yet, these beneficial effects can be hijacked by disease-causing mutations in FGF receptor (FGFR) during embryogenesis. By studying dominant FGFR2 mutations that are germline in bent bone dysplasia syndrome (BBDS), we reveal a mechanistic connection between FGFR2, ribosome biogenesis, and cellular stress that links cell fate determination to disease pathology.

Ribosome biogenesis is dynamically regulated during osteoblast differentiation.

Changes in ribosome biogenesis are tightly linked to cell growth, proliferation, and differentiation. The rate of ribosome biogenesis is established by RNA Pol I-mediated transcription of ribosomal RNA (rRNA). Thus, rRNA gene transcription is a key determinant of cell behavior.

Preformed gelatin microcryogels as injectable cell carriers for enhanced skin wound healing.

Unlabelled: Wound dressings of cell-laden bulk hydrogel or scaffold were mainly applied for enhanced cell engraftment in contrast to free cell injection. However, dressing of cells laden in biomaterials on wound surface might not effectively and timely exert functions on deep or chronic wounds where insufficient blood supply exists. Previously, we developed injectable gelatin microcryogels (GMs) which could load cells for enhanced cell delivery and cell therapy.